SATURDAY FORUM.SATURDAY FORUM.1414/ 05/ 2005/ 05/ 2005

Dr.Shantha Herath.Dr.Shantha Herath.

Mr. K.M.K. 63yrs old retired teacher from WalapaneMr. K.M.K. 63yrs old retired teacher from Walapane c/o slurring in speechc/o slurring in speech weakness of the R/S of the body / 4/12weakness of the R/S of the body / 4/12

H.P.C H.P.C -pt was well before 4/12. Then he developed slurring of -pt was well before 4/12. Then he developed slurring of speech which was gradually progressive. After about 1/12,speech which was gradually progressive. After about 1/12, he noticed that increase salivation too and also developedhe noticed that increase salivation too and also developed difficulty in swallowing and sometimes nasal regurgitation difficulty in swallowing and sometimes nasal regurgitation of fluid.of fluid. Sametime he noticed some weakness of the R/S ofSametime he noticed some weakness of the R/S of the body which was also progressive and about 3/12 laterthe body which was also progressive and about 3/12 later he noticed that his left arm also become weak.he noticed that his left arm also become weak. No convulsions, no numbness of the bodyNo convulsions, no numbness of the body No mouth ulcers,No mouth ulcers,

No impairment of vision or double visionNo impairment of vision or double vision No retention or incontinence of urine or fecesNo retention or incontinence of urine or feces no chronic cough,or haemoptysisno chronic cough,or haemoptysisP.M.HP.M.H. . Diagnosed case of intermittent AF and calcific aortic valveDiagnosed case of intermittent AF and calcific aortic valve No D.M, HTNo D.M, HT No H/O polio myolitesNo H/O polio myolites No H/O musculo-skeletal disorderNo H/O musculo-skeletal disorder

Drug/H Drug/H on warfarin for AF on warfarin for AF no other drug history availableno other drug history available Social H.Social H. - lives with chilren - lives with chilren - monthly income 6000/=- monthly income 6000/= - adequate diet- adequate diet - non-smoker- non-smoker -non alcoholic-non alcoholic

Family- History - 4 childrenFamily- History - 4 children - 3 boys- 3 boys - wife died 5 years back- wife died 5 years back - all are healthy- all are healthy - no similar illness- no similar illness

SummarySummary- - Mr. K.M.K. 63yrs old retired teacher Mr. K.M.K. 63yrs old retired teacher

presented with presented with difficulty in speech, increase difficulty in speech, increase

salivation and weakness of salivation and weakness of both upper limbs and R/ lower both upper limbs and R/ lower

limb. For 4/12 with dysphagia and nasal limb. For 4/12 with dysphagia and nasal regurgitation. With regurgitation. With no convalsions, no numbness of the no convalsions, no numbness of the body , no visual impairment or diplopia body , no visual impairment or diplopia or sphincter disturbances. or sphincter disturbances.

EXAMINATIONEXAMINATION

G/E. G/E. ill lookingill looking

Conscious and rational Conscious and rational

No ptosis, pupils normalNo ptosis, pupils normal

Not pale, not icteric Not pale, not icteric

No Cyanosis No Cyanosis

No generalized lymphadenopathyNo generalized lymphadenopathy

No clubbing or splinter haemorrageNo clubbing or splinter haemorrage

No peripheral edemaNo peripheral edema

C.V.S BP 140/85 mmHgC.V.S BP 140/85 mmHg

Pulse 100/mm, irregularly irregularPulse 100/mm, irregularly irregular

No m No m

R.SR.S Clinically normal Clinically normal

AbdAbd Clinically normal Clinically normal

C.N.S. Higher functions normal C.N.S. Higher functions normal

CRANIAL NERVECRANIAL NERVE

I – VIII – Clinically normal I – VIII – Clinically normal

- Spastic tongue with fasciculation Spastic tongue with fasciculation - Bilateral palatal weakness +Bilateral palatal weakness +- Slurring of Speech +Slurring of Speech +

UPPER LIMBSUPPER LIMBS - Wasting of small muscle of both hands Wasting of small muscle of both hands

and muscle of forearm with fasciculationand muscle of forearm with fasciculation- Deep guttering of dorsum of both hands. Deep guttering of dorsum of both hands.

R/UL R/UL L/ULL/UL

Power Power 4/54/5 4/54/5

Tone Tone Slightly Slightly Slightly Slightly

Reflexes Reflexes BJ- Brisk BJ- Brisk Brisk Brisk

TJ- Brisk TJ- Brisk Brisk Brisk

SJ- Brisk SJ- Brisk Brisk Brisk

LOWER LIMBSLOWER LIMBS

R/LCR/LC L/LCL/LC

PowerPower 4/54/5 5/55/5

Tone Tone IncreaseIncrease NormalNormal

ReflexesReflexes KJ- Brisk KJ- Brisk Normal Normal

AJ- BriskAJ- Brisk NormalNormal

Planter- ?Planter- ? Normal Normal

No sensory involvement No sensory involvement Cerebellar functions normal Cerebellar functions normal

SUMMARYSUMMARY - Mr. K.M.K. 63yrs old retired teacher presented with - Mr. K.M.K. 63yrs old retired teacher presented with

slurring of speech, and weakness of both upper slurring of speech, and weakness of both upper limbs and R/ lower limb. For 4/12 with dysphagia and limbs and R/ lower limb. For 4/12 with dysphagia and nasal regurgitation and wasting of both forearms and nasal regurgitation and wasting of both forearms and hand muscles with fasciculation and also spastic tongue hand muscles with fasciculation and also spastic tongue with dysarthria. With no convulsions, no numbness of the with dysarthria. With no convulsions, no numbness of the body , no visual impairment or diplopia or sphincter body , no visual impairment or diplopia or sphincter disturbances. disturbances.

Motor Neuron DiseaseMotor Neuron Disease

-Selective loss of functions of lower/upper motor Selective loss of functions of lower/upper motor neuronsneurons- The voluntary muscles of limbs and bulbar region - The voluntary muscles of limbs and bulbar region - Family of diseases- Family of diseases- In practice – DDS – on – clinically, electro - In practice – DDS – on – clinically, electro physiologicallyphysiologically-In clinical diagnosisIn clinical diagnosis- -Anatomical differentiation is augmented by-Anatomical differentiation is augmented by - age of onset- age of onset -rate of deterioration-rate of deterioration - familial occurrence- familial occurrence Sensation and cognition – normalSensation and cognition – normal

-Lower motor neuron diseaseLower motor neuron disease - Wasting- Wasting - Fasciculation- Fasciculation- - Flaccid weakness- Flaccid weakness

-

* * * Tendon reflexes are retained until late stage* Tendon reflexes are retained until late stage * Nerve conduction studies to exclude peripheral * Nerve conduction studies to exclude peripheral

neuropahtyneuropahty

* Muscle biopsy –* Muscle biopsy – to exclude myopathy.to exclude myopathy.

eg.-progressive proximal limb eg.-progressive proximal limb

weakness weakness -bulbar -bulbar weaknessweakness

upper motor neuron diseases upper motor neuron diseases - spasticity- spasticity - clonus- clonus

* extensor planter response* extensor planter response - weakness- weakness *abd – reflex often preserved.*abd – reflex often preserved. * Sphincters and sexual functions usually preserved* Sphincters and sexual functions usually preserved

M.N.D.-incurableM.N.D.-incurable - but- help disabilities- but- help disabilities Eg: -malnutrition due to dysphasiaEg: -malnutrition due to dysphasia - NG tube- NG tube - gastrostomy- gastrostomy - assisted ventilation- assisted ventilation -spasticity -spasticity drugs – Beclofendrugs – Beclofen DantroleneDantrolene diazapamdiazapam

- Weel chairs- Weel chairs - Amitriptallin for emotional lability of bulbar palsy- Amitriptallin for emotional lability of bulbar palsy

Classification of M.N.DClassification of M.N.D* - A.L.S* - A.L.S - sporadic- sporadic - familial adult onset (A/D)- familial adult onset (A/D) - familial juvenile onset (A/R)- familial juvenile onset (A/R) * Lower motorneuron disease* Lower motorneuron disease - proximal hereditary M.N.O- proximal hereditary M.N.O

-acute infantile (warding Hoffman)type-acute infantile (warding Hoffman)type - chronic childhood form- chronic childhood form (Kugelburg-welander) A/R(Kugelburg-welander) A/R - adult onset-AR- adult onset-AR - adult onset-AD- adult onset-AD - hereditary bulbar palsy- hereditary bulbar palsy - with deafness- with deafness - without deafness- without deafness - X linked bulbospinal neuropathy- X linked bulbospinal neuropathy (Kennady syndrome)(Kennady syndrome) - multifocal motor neuropathy- multifocal motor neuropathy - post polio syndrome- post polio syndrome

- post irradiation syndrome- post irradiation syndrome * Upper motor neuron syndrome* Upper motor neuron syndrome - primary lateral scleroses- primary lateral scleroses - hereditary spastic paraplegia (AD)- hereditary spastic paraplegia (AD) - lathyrism- lathyrism

Upper/Lower M.N.DUpper/Lower M.N.D ALS - male>femaleALS - male>female - with age- with age - unusual before 5- unusual before 5 thth decad decad - unknown cause- unknown cause - ass c dementia and parkinsonism- ass c dementia and parkinsonism

Pathology-Pathology- - L.M. neuron loss in the cord and brain stem- L.M. neuron loss in the cord and brain stem - bumina bodies in surviving neuron- bumina bodies in surviving neuron - Betz cells in motor cortex- Betz cells in motor cortex - pyramidal tract degeneration- pyramidal tract degeneration Clinical features-Clinical features- - balbar-spinal symptoms- balbar-spinal symptoms Prognosis- median survival range 20/12Prognosis- median survival range 20/12 Rx - Rx - - speech therapist- speech therapist - occupational therapist- occupational therapist - social worker- social worker - physician- physician

THANK YOU.THANK YOU.